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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
1
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pubmed:dateCreated |
2007-5-15
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pubmed:abstractText |
Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) results from a deficiency of CLN3, a protein recently identified within detergent-resistant membranes (DRMs). To study the function of CLN3 within these domains we isolated DRMs from control and JNCL-brain and noted that JNCL-derived DRMs are less buoyant than control. Analysis of DRM phospholipids derived from JNCL-brain revealed a reduction of bis(monoacylglycerol)phosphate. Metabolic labeling of JNCL-fibroblasts demonstrated a reduction in the synthesis of bis(monoacylglycerol)phosphate which was restored following complementation with wild-type-CLN3, substantiating our initial observation in brain. Metabolic labeling of cell lines overexpressing wild-type-CLN3 resulted in increased bis(monoacylglycerol)phosphate synthesis, while overexpression of mutant CLN3-L170P decreased bis(monoacylglycerol)phosphate synthesis. These data illustrate a new finding, a strong correlation between CLN3 protein expression and synthesis of bis(monoacylglycerol)phosphate.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10191112,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10191116,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10428067,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10508524,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10649502,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10749980,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10856224,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10924275,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-10942779,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-11104761,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-11278374,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-11308026,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-11707436,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-11722572,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-11791207,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-11997263,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-12059962,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-12065580,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-12645080,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-12706816,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-14660799,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-14689449,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-15094366,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-15706348,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-16212488,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-16251196,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-16508006,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-17036287,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-5483450,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-7139611,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-7553855,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-8627293,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-9417773,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-9662406,
http://linkedlifedata.com/resource/pubmed/commentcorrection/17482562-9804787
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Jun
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pubmed:issn |
0006-291X
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
22
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pubmed:volume |
358
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
111-6
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pubmed:dateRevised |
2011-9-26
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pubmed:meshHeading |
pubmed-meshheading:17482562-Brain,
pubmed-meshheading:17482562-Cells, Cultured,
pubmed-meshheading:17482562-Fibroblasts,
pubmed-meshheading:17482562-Humans,
pubmed-meshheading:17482562-Intracellular Membranes,
pubmed-meshheading:17482562-Lysophospholipids,
pubmed-meshheading:17482562-Membrane Glycoproteins,
pubmed-meshheading:17482562-Membrane Microdomains,
pubmed-meshheading:17482562-Molecular Chaperones,
pubmed-meshheading:17482562-Monoglycerides,
pubmed-meshheading:17482562-Mutation,
pubmed-meshheading:17482562-Neuronal Ceroid-Lipofuscinoses
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pubmed:year |
2007
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pubmed:articleTitle |
A novel role of the Batten disease gene CLN3: association with BMP synthesis.
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pubmed:affiliation |
Committee on Molecular Metabolism and Nutrition, Biological Sciences Division, The University of Chicago, Chicago, IL, USA. hobertj@ccf.org
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pubmed:publicationType |
Journal Article,
Research Support, N.I.H., Extramural
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